Ambulatory blood pressure and hypertension control in children with autosomal recessive polycystic kidney disease: clinical experience from two central European tertiary centres

Abstract

Objective : Arterial hypertension is a common complication in patients with autosomal recessive polycystic kidney disease (ARPKD), occurring in 33-75% of children when measured by office blood pressure (OBP). Ambulatory blood pressure monitoring (ABPM) is a superior tool for investigating blood pressure relative to OBP. The aim of our study was to investigate the prevalence and control of hypertension in children with ARPKD based on ABPM.Methods: This retrospective study evaluated 36 children with ARPKD and at least one ABPM performed in two our tertiary paediatric nephrology centres and 29 children with at least two ABPM. Ambulatory hypertension was defined as mean daytime or night-time BP at least 95th percentile or use of antihypertensives and controlled hypertension as normal ambulatory BP in children on antihypertensive drugs. Results:The first ABPM study revealed ambulatory hypertension in 94% of children. Untreated or uncontrolled ambulatory hypertension was diagnosed in 67% and controlled hypertension in only 28%. Masked hypertension was found in 5.5% and white-coat hypertension in 14%. The last ABPM study revealed ambulatory hypertension in 86% (all 86% hypertensive children on drugs, i.e. no untreated hypertension), the prevalence of controlled hypertension increased to 59%. Masked hypertension was detected in 8.3% and whitecoat hypertension in 10%. Ambulatory blood pressure correlated neither with kidney length nor with glomerular filtration rate. Echocardiography demonstrated left ventricular hypertrophy (LVH) in 27% of children at the time of their first ABPM. Conclusion:The prevalence of ambulatory hypertension is very high in children with ARPKD, while the control of hypertension improves over time.

Autosomal dominant and autosomal recessive polycystic kidney disease: hypertension and secondary cardiovascular effect in children

“…The median age at the first ABPM was 4.4 years and the average interval between the first and the last recording was 5 years. Confirming previous reports (Dell et al, 2016), the initial evaluation showed hypertension in 94% of patients, of which only one-third were well controlled with antihypertensive therapy (Seeman et al, 2022). At the last evaluation, the prevalence of hypertension remained stable (86%), but two-third of patients were now adequately treated.…”

“…The estimated prevalence of hypertension in published studies ranges from 33% to 75% (Deget et al, 1995;Zerres et al, 1996;Guay-Woodford and Desmond, 2003;Bergmann et al, 2005;Dell, 2011;Guay-Woodford et al, 2014). Hypertension develops even in the first weeks of life and is often difficult to control, requiring early combined therapy with different antihypertensive drugs (Deget et al, 1995;Zerres et al, 1996;Guay-Woodford et al, 2014;Chinali et al, 2019;Seeman et al, 2022). Hypertension is not always associated with kidney failure.…”

“…Seeman et al have also evaluated cardiac geometry in ARPKD children using two-dimensional echocardiography. They observed LVH in 30% of patients, including some patients that had good blood pressure control (Seeman et al, 2022).…”

Masked Hypertension in Healthy Children and Adolescents: Who Should Be Screened?

“…In the autosomal recessive form of polycystic kidney disease (ARPKD), MH has been found in a most recent study in only 5% of patients, apparently because of the high prevalence of sustained hypertension evident also by office BP measurements (94%) [ 19 ].…”

Kidney concentrating capacity in children with autosomal recessive polycystic kidney disease is linked to glomerular filtration and hypertension

“…The demographic and clinical data, antihypertensive medication, symptoms, and complications of children with ARPKD are shown in Table 1. Thirteen patients were described in our earlier paper on ambulatory blood pressure in ARPKD [11].…”